Sturge-Weber syndrome is also known as encephalotrigeminal angiomatosis. Which feature is characteristic?

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Multiple Choice

Sturge-Weber syndrome is also known as encephalotrigeminal angiomatosis. Which feature is characteristic?

Sturge-Weber syndrome revolves around abnormal capillary-venous vessels in the brain along with a facial vascular birthmark. The hallmark is a port-wine stain on the face in the trigeminal nerve distribution that is typically matched on the same side by leptomeningeal angiomatosis. This combination explains the neurological issues seen, such as seizures and focal deficits, and imaging often shows the characteristic tram-track calcifications from the leptomeningeal vascular malformations. The other options point to different neurocutaneous conditions: bilateral vestibular schwannomas are classic for neurofibromatosis type 2, Ash-leaf spots for tuberous sclerosis, and cafe-au-lait spots for neurofibromatosis type 1.

Sturge-Weber syndrome revolves around abnormal capillary-venous vessels in the brain along with a facial vascular birthmark. The hallmark is a port-wine stain on the face in the trigeminal nerve distribution that is typically matched on the same side by leptomeningeal angiomatosis. This combination explains the neurological issues seen, such as seizures and focal deficits, and imaging often shows the characteristic tram-track calcifications from the leptomeningeal vascular malformations. The other options point to different neurocutaneous conditions: bilateral vestibular schwannomas are classic for neurofibromatosis type 2, Ash-leaf spots for tuberous sclerosis, and cafe-au-lait spots for neurofibromatosis type 1.

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